Alveolar soft part sarcoma of the head and neck

The twelfth case (in 25 years of literature) of alveolar soft part sarcoma of the head and neck is presented. It represents the seventh case to occur in the base of tongue and the second to be associated with pregnancy. The average age of onset is in the second decade with a 2:1 female preponderance and a marked tendency to right-sided occurrence. Treatment with surgery and/or radiotherapy is not curative. Local recurrence is common as are distant metastases, usually to lung, brain or bone. Theoretically, chemotherapy may offer better control of disease but few agents have shown dramatic response. The characteristics of the tumors are discussed and various chemotherapeutic agents are reviewed.